The natural history of primary biliary cirrhosis (PBC) has improved significantly over the last two decades. Most patients are diagnosed with asymptomatic PBC (a-PBC). The prognosis of a-PBC is usually better than that of symptomatic PBC (s-PBC). Among a-PBC patients, some remain asymptomatic, whereas others progress to s-PBC. The prognosis of s-PBC is still poor and the main cause of death in PBC is liver failure. Other complications, such as esophageal varices and hepatocellular carcinoma, also affect the prognosis of PBC patients. Ursodeoxycholic acid treatment improves the prognosis of PBC patients in the early stage. There seems to be several types of PBC progression.